History

The major symptoms of exocrine pancreatic insufficiency (EPI) include steatorrhea and weight loss. The most common symptomatic complaint is diarrhea, which is frequently watery, reflecting the osmotic load received by the intestine.

Steatorrhea is the result of fat malabsorption and is characterized by pale, bulky, and malodorous stools. These stools often float on top of the toilet water with oily droplets and are difficult to flush.

Weight loss and fatigue are common and may be pronounced; however, patients may compensate by increasing their caloric consumption, and as a result, weight loss from malabsorption may be masked. The weight loss may be compounded by an underlying disease involving the intestine (eg, celiac disease or Crohn disease).

Flatulence and abdominal distention are caused by bacterial fermentation of unabsorbed food substances, which releases gaseous products such as hydrogen and methane. Flatulence often causes uncomfortable abdominal distention and cramps.

Edema may result from hypoalbuminemia caused by chronic protein malabsorption; loss of protein into the intestinal lumen can cause peripheral edema. With severe protein depletion, ascites may develop.

Anemia resulting from malabsorption can be either microcytic (related to iron deficiency) or macrocytic (related to vitamin B-12 deficiency). Anemia may also be associated with the underlying disease causing EPI. For instance, iron deficiency anemia is often a manifestation of celiac disease. Ileal involvement in Crohn disease or ileal resection can cause megaloblastic anemia due to vitamin B-12 deficiency.

Bleeding disorders are usually a consequence of vitamin K malabsorption and subsequent hypoprothrombinemia. Ecchymosis usually is the manifesting symptom, though melena and hematuria may occur on occasion.

Metabolic bone disease caused by vitamin D deficiency can result in osteopenia or osteomalacia. In severe cases, bone pain and pathologic fractures occur with low calcium levels leading to secondary hyperparathyroidism.

Neurologic manifestations can result from electrolyte disturbances (eg, hypocalcemia and hypomagnesemia) and can lead to tetany. Malabsorption of certain vitamins can cause generalized motor weakness (pantothenic acid and vitamin D), peripheral neuropathy (thiamine), loss of a sense of vibration and position (cobalamin), night blindness (vitamin A), or seizures (biotin).

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Physical Examination

Signs of weight loss, muscle wasting, and loss of subcutaneous fat are often noted.

On abdominal examination, orthostatic hypotension may be present. The abdomen may be distended, and bowel sounds may be hyperactive. In severe hypoproteinemia, ascites may be present. Peripheral edema may be observed.

On dermatologic examination, pale skin may reflect anemia. Ecchymoses due to vitamin K deficiency may be apparent. Dermatitis herpetiformis, erythema nodosum, and pyoderma gangrenosum may be noted. Pellagra, alopecia, or seborrheic dermatitis may be present. Examination of the mouth may reveal cheilosis, glossitis, or aphthous ulcers.

On neurologic examination, motor weakness, peripheral neuropathy, or ataxia may be present. The Chvostek sign or the Trousseau sign may be noted as a consequence of hypocalcemia or hypomagnesemia.

Complete replacement of the pancreas with cystic disease and intrahepatic biliary dilation caused by extrinsic compression of the common bile duct. Note also the renal cysts and masses. This patient had exocrine pancreatic insufficiency. Image courtesy of Wikimedia Commons.